可调压分流管治疗颅内静脉异常回流所致高颅压性脑积水

目的 探讨可调压分流管治疗先天性颅内静脉回流异常所致脑积水的应用价值.方法 2例男性患儿行CT、MRI提示脑室系统扩张,MRV及DSA检查发现颅内静脉窦广泛闭塞.腰穿压力显著升高.手术均采用强生可调压分流管行脑室-腹腔体外可调压分流术治疗.结果 2例患者术中均将分流阀压力设定为200 mmH2O,术后逐步调低,调低至180 mmH2O均出现不同程度低颅压症状,调高后症状缓解,2例患者术后智力及运动功能显著提高,复查CT提示脑室减小.结论 先天性颅内静脉异常回流导致的高颅压性脑积水非常少见,治疗经验甚少.可调压分流显然是治疗颅内静脉回流导致高颅压性脑积水一种最佳治疗方法,对压力调节,需通过较长时间多次调节来逐步达到一个合适的个体压力.     

可调压分流管治疗颅内静脉异常回流所致高颅压性脑积水

目的 探讨可调压分流管治疗先天性颅内静脉回流异常所致脑积水的应用价值.方法 2例男性患儿行CT、MRI提示脑室系统扩张,MRV及DSA检查发现颅内静脉窦广泛闭塞.腰穿压力显著升高.手术均采用强生可调压分流管行脑室-腹腔体外可调压分流术治疗.结果 2例患者术中均将分流阀压力设定为200 mmH2O,术后逐步调低,调低至180 mmH2O均出现不同程度低颅压症状,调高后症状缓解,2例患者术后智力及运动功能显著提高,复查CT提示脑室减小.结论 先天性颅内静脉异常回流导致的高颅压性脑积水非常少见,治疗经验甚少.可调压分流显然是治疗颅内静脉回流导致高颅压性脑积水一种最佳治疗方法,对压力调节,需通过较长时间多次调节来逐步达到一个合适的个体压力.     

可调压式脑室-腹腔分流管治疗脑积水

目的探讨可调压分流式脑室-腹腔分流管治疗脑积水的有效性和安全性。方法自2007年8月至2010年9月,采用可调压分流管对18例脑积水患者行脑室-腹腔分流术,同时期有61例脑积水患者使用固定压力分流管手术,比较用两种分流管手术的治疗效果。结果可调压管分流组未发生硬膜下积液或硬膜下血肿,无因为脑脊液分流过度或不足而需再次手术治疗者;2例患者术后出现堵管或感染。固定压力管分流组发生颅内血肿或积液4例;堵管或感染5例,两组之间无统计学差异(P>0.05)。结论可调压分流管对脑积水的治疗具有很好的安全性,更符合脑脊液的循环压力需要,并在减少分流过度和不足方面优于不可调压分流管。     

脑室-腹腔分流术治疗婴幼儿脑积水的疗效分析简

目的总结可调压式分流管治疗婴幼儿脑积水的临床经验,探讨其临床应用价值。方法回顾性分析29例植入可调压式分流管治疗不同类型婴幼儿脑积水的临床资料。患儿均行脑室-腹腔分流术,术中根据侧脑室的测压结果设定分流阀的初始压力,术后根据患儿症状体征的改善情况及影像学结果调整分流阀的压力。结果术后随访3—48个月,患儿临床症状均明显缓解或消失,CT检查显示脑室均较术前缩小。术后感染2例,分流管梗阻3例。结论可调压式分流管具有个体化设定分流阀压力的优点,可有效避免术后分流过度和分流不足的出现,是治疗婴幼儿脑积水的首选方法。     

脑室-腹腔分流术治疗婴幼儿脑积水的疗效分析

目的总结可调压式分流管治疗婴幼儿脑积水的临床经验,探讨其临床应用价值。方法回顾性分析29例植入可调压式分流管治疗不同类型婴幼儿脑积水的临床资料。患儿均行脑室-腹腔分流术,术中根据侧脑室的测压结果设定分流阀的初始压力,术后根据患儿症状体征的改善情况及影像学结果调整分流阀的压力。结果术后随访3~48个月,患儿临床症状均明显缓解或消失,CT检查显示脑室均较术前缩小。术后感染2例,分流管梗阻3例。结论可调压式分流管具有个体化设定分流阀压力的优点,可有效避免术后分流过度和分流不足的出现,是治疗婴幼儿脑积水的首选方法。     

面部血管痣合并脑积水的颅内静脉异常回流三例

目的 探讨面部血管痣合并脑积水患者的颅内静脉异常回流特征及其临床表现.方法 结合文献复习,回顾性分析3例面部血管痣合并先天脑积水患儿的脑血管造影资料及临床表现.结果 3例患儿均有多处广泛的静脉窦闭塞且影响全脑静脉回流,颅内静脉回流主要经异常扩张的导静脉或代偿形成的侧副路静脉反流至头面部,引起双侧头面部及颈部静脉扩张.结论 广泛的颅内静脉窦闭塞导致颅内异常的静脉回流是面部血管痣患儿发生脑积水的主要原因,而这种静脉窦闭塞及异常回流静脉的形成很可能源于胎儿胚胎期发育缺陷.     

天幕区硬脑膜动静脉瘘压迫导水管导致阻塞性脑积水

目的报道1例以阻塞性脑积水为首发表现的硬脑膜动静脉瘘(DAVF)。方法一男性52岁病人因行走不稳、尿失禁入院,CT和MR发现阻塞性脑积水,天幕附近存在异常血管影。脑血管造影诊断为天幕区DAVF,脑积水由扩张的引流静脉压迫中脑导水管所致。病人接受了血管内栓塞和脑室-腹腔分流手术。结果大部分闭塞了DAVF,脑积水症状消失。结论DAVF扩张的引流静脉压迫中脑导水管导致阻塞性脑积水极其罕见,这是文献中第2例有详细报道的此类病例;采用血管内方法闭塞病灶并行脑室-腹腔分流手术是合适的治疗方法。     

裂隙脑室综合征的临床治疗探讨:31例报告

目的分析裂隙脑室综合征临床过程和病理生理学机制,探讨裂隙脑室综合征的适当临床诊疗方法。方法回顾性分析31例裂隙脑室综合征患者的临床特点、术式选择以及预后;其中6例行更换分流管系统或重新脑室腹腔分流,25例行腰大池-腹腔分流。结果术后所有患者头痛、恶心呕吐症状完全缓解,视力损害的症状得到明显改善,预后良好;3例术前检查发现有横窦狭窄或闭塞的,术后半年复查头MRV显示横窦重新开放。结论腰大池-腹腔分流术相对简易、安全,适用于裂隙脑室综合征患者;蛛网膜囊肿或者脑积水分流手术应选择可调压抗虹吸的分流管系统,利于后期调整合适压力,减少裂隙脑室综合征的发生率;裂隙脑室综合征可以出现颅内静脉窦变窄,随着颅压恢复正常后,静脉窦狭窄可以消失。     

IGCTs所致梗阻性脑积水远期分流装置移除的可行性探讨

目的 探讨可调压抗虹吸分流装置在颅内生殖细胞肿瘤(IGCTs)所致脑积水治疗中的意义及远期移除分流装置的可行性。方法 重庆医科大学附属儿童医院神经外科2013年1月—2017年12月间42例拟诊为IGCTs伴梗阻性脑积水患儿,均采用可调压抗虹吸分流装置行脑室腹腔分流术(VPS),术后予以行诊断性化疗;回顾性分析其中20例在肿瘤达到完全缓解后行分流装置移除术的诊疗过程。结果 行VPS术后,20例患儿的高颅压症状均获得显著缓解,无症状加重者。术后复查CT或MRI显示脑室系统正常;眼底检查未见明显损害情况,术前视力模糊者术后视力逐渐恢复正常,现视力与同龄人相仿。治疗过程中未见分流装置堵塞、感染病例,予以诊断性化疗,肿瘤获得完全缓解,6～12个月后行分流装置夹闭或移除术,随访36～92个月,20例患者均未出现颅内高压症状。至随访结束时均已入学,生活质量未见明显影响。结论 IGCTs所致梗阻性脑积水行VPS早期可以快速降低颅压,缓解颅内高压症状,降低颅内压持续升高而发生脑疝的风险。术后经诊断性化疗,在肿瘤获得完全缓解后,可行分流装置移除术,降低分流装置堵塞、感染、断裂等风险,避免患儿终生留置体内...     

脑室-腹腔分流术后分流过度45例临床分析

脑积水目前最常采用的治疗方法是行侧脑室腹腔分流水,但其并发症较多,其中分流过度是造成分流手术失败的原因之一。文献报道也较少,1992～1997年收治各种脑积水45例,全部行侧脑室腹腔分流术,就其中分流过度者四例进行了讨论,参阅了国内史氏,及国外少部分资料,就分流过度的诊断。如颅内血肿,缝隙样脑室综合症,低颅压综合症,颅缝早闭,小头畸形,导水管闭塞等及分流过度与选择分流管压力限值的关系,分流过度与按压分流泵的关系等进行了讨论,提出注重鉴别分流过度所致颅内血肿与手术穿刺损伤所致颅内血肿,提出颅内压监护在分流术前的重要性,并可对现行的分流管腹腔端裂隙阀门进行修改的意见,主张不能按压分流泵,对改善分流管设计提出了新的想法。     

可调压分流管治疗颅内静脉异常回流所致高颅压性脑积水

目的 探讨磁共振相位电影对比成像法在内镜下导水管成形术治疗导水管梗阻性脑积水的应用价值.方法 对23例诊断为导水管梗阻性脑积水的患者,术前常规采用磁共振相位电影对比法进一步确诊;手术采用电子软性神经内镜下导水管成形术,术中对导水管阻塞程度进行评估.术后1周及随访均采用磁共振相位电影对比法复查来测量导水管脑脊液流速流量以确定导水管是否开通.结果 23例术前磁共振相位电影对比法未见导水管脑脊液流动患者,术中见导水管完全闭塞或直径小于1 mm2;23例患者成形术均获成功,导水管扩张平均在4 mm左右,术后1周电影成像检查导水管平均流速为(4.74±1.77)cm/s,在随访期间,2例再次出现颅高压症状的患者,电影成像显示导水管未见脑脊液流动,二次内镜下探查见导水管重新闭塞.结论 磁共振相位电影对比法通过测量导水管内脑脊液流速流量来精确判断导水管开通情况,可以作为导水管梗阻性脑积水术前诊断及导水管成形术后疗效判断及随访的重要工具.

Abstract：

Objective To evaluate the application of phase-contrast cine magnetic resonance imaging (MRI) in endoscopic aqueductoplasty for patients with obstructive hydrocephalus. Methods The clinical diagnosis of hydrocephalus due to aqueduct obstruction in 23 patients was confirmed by phasecontrast cine MRI examination. The patients were treated with endoscopic aqueductoplasty. MRI was repeated during follow- up period. The cerebrospinal fluid (CSF) flow velocity in aqueduct was measured to determine whether the aqueduct was obstructed. Results The Results of phase -contrast cine MRI examinations indicated that there was no CSF flow in aqueduct in any patient. Aqueductoplasty was successfully performed in all patients. After one week, the Results of phase - contrast cine MRI examinations showed an average CSF flow velocity of (4.74 ± 1.77) cm/s. During follow - up period, intracranial hypertension recurred in two patients in whom CSF flow was not seen inside the aqueduct by phase - contrast cine MRI scan and the aqueduct re - occlusion was revealed by endoscopic exploration. Conclusions By measuring CSF flow velocity, phase - contrast cine MRI could accurately identify whether the aqueduct is obstructed. It should play an important role in the diagnosis of obstructive hydrocephalus and evaluation of theeffectiveness of aqueductoplasty, and it could be used for follow - up evaluation as well.     

Occurrence of subdural hematoma and resolution of gait disturbance in a patient treated with shunting for normal pressure hydrocephalus

A 66-year-old man with gait disturbance was diagnosed with normal pressure hydrocephalus (NPH) and treated with ventriculoperitoneal shunting using a programmable valve. The valve ultimately set at a pressure of 40 mmH₂O after higher settings no longer relieved symptoms. However, this pressure setting was excessively low and was associated with occurrence of bilateral subdural hematomas. Paradoxically, this event was associated with stable improvement of gait. Our patient's gait disturbance was unassociated with muscle weakness, spasticity, cerebellar ataxia, or Romberg's sign, and, therefore, was consistent with a frontal gait disorder. Cerebral cortical blood flow as measured after shunting by single photon emission computed tomography (SPECT) was slightly increased from the value before shunting, possibly because of intracranial hypotension related to the valve setting. Lasting improvement of gait in our case may be a result of increased blood flow in the supplementary motor area (SMA).     

Sturge-Weber syndrome variant with atypical intracranial findings: case report

Sturge-Weber syndrome is characterized by a facial port-wine nevus, leptomeningeal angiomatosis, and glaucoma; it is commonly complicated by epilepsy and hemiparesis. We present a patient with a head and neck port-wine nevus, glaucoma, abnormalities of the intracranial deep veins, and untreated communicating hydrocephalus. The patient lacks any radiologic or clinical evidence of cerebral leptomeningeal angiomatosis. Considering that intracranial venous anomalies also are likely compatible with the embryologic explanation of Sturge-Weber syndrome, this child can serve as an unusual example of Sturge-Weber syndrome type II.     

Over-shunting associated myelopathy

Intracranial hypotension typically presents following cerebrospinal fluid (CSF) leak, but can be induced by CSF diversion. Classically, patients present with positional headache, but less common symptoms include neck pain and cranial nerve palsies. To our knowledge, the neurosurgical literature contains six reports of patients with symptomatic cervical, epidural venous plexus engorgement as the result of CSF shunting. The patient presented herein is a 26-year-old woman with shunt-dependent, congenital hydrocephalus. She presented with rapidly progressive cervical myelopathy following ventriculoperitoneal shunt revision. Imaging revealed engorgement of the cervical epidural venous plexus and mass effect on the cervical spinal cord. “Over-shunting associated myelopathy” is a rare complication of CSF diversion that should be familiar to physicians who routinely evaluate patients with intracranial shunts.     

Hydrocephalus caused by increased intracranial venous pressure: A clinicopathological study

A child developed progressive communicating nonobstructive hydrocephalus as a result of increased intracranial venous pressure. The child had been treated for congenital heart disease at age 3 weeks by surgical creation of an anastomosis between his superior vena cava and right pulmonary artery. Although his cardiac symptoms were alleviated, intracranial venous hypertension resulted, giving rise to progressive head enlargement and other signs of hydrocephalus. Postmortem examination at age 3 years disclosed no other lesions that could have caused the hydrocephalus. Increased intracranial venous pressure can lead to either pseudotumor cerebri or hydrocephalus, the former in children 3 years or older, the latter in infants 18 months or less. Possible mechanisms accounting for these differences are discussed.     

Learning disability and impairment of synaptogenesis in HTX-rats with arrested shunt-dependent hydrocephalus

Using HTX-rats with congenital hereditary hydrocephalus, we used neuropathological methods, including quantitative Golgi study and neurobehavioral evaluation, to investigate the following problems. (1) What kind of damage does congenital hydrocephalus cause to developing brain tissue? (2) How much can the damage be repaired by ventriculoperitoneal shunting if performed at 4 weeks of age, enabling 4-week-old hydrocephalic rats to survive beyond sexual maturation? (3) What is the status of learning ability of long-term surviving rats with arrested shunt-dependent hydrocephalus? The findings of our study suggest that congenital hydrocephalus impairs the development and formation of the dendrites and spines of the cerebrocortical neurons. Following ventriculoperitoneal shunting, we confirmed that rats with arrested shunt-dependent hydrocephalus demonstrated learning disability in a light-darkness discrimination test using a Y-maze. The development of the dendrites and spines of the cerebrocortical neurons seemed to take place to some degree after shunting, but normal spine density could not be restored. Also suggested was a possible relationship between learning disability and a decrease in spine density, i.e., impairment of synaptogenesis.     

Management of tectal glioma in childhood

Tectal glioma is a topographical diagnosis including tumors of different histology, mainly low-grade astrocytomas. Clinical symptoms are usually associated with increased intracranial pressure. This report discusses the management of this rare tumor in children. Clinical charts of 12 children with tectal glioma treated in our department between 1976 and 2001 were retrospectively reviewed. The mean age at the time of diagnosis was 6.75 years (range, 4 weeks to 16 years). The duration between first symptoms and the diagnosis of tectal glioma was in the range of 2 days to 9 years. Ten patients presented with symptoms associated with increased intracranial pressure, one patient presented with ataxia, and in one case tectal glioma was an incidental finding. First-line therapy was endoscopic third ventriculostomy in 5 cases (42%), ventriculoperitoneal shunting in 6 cases (50%), and combined partial tumor resection and shunting in one case. Histology was obtained in 5 cases (low-grade astrocytoma, n = 4; ependymoma, n = 1). All patients had good neurologic function at the end of follow-up. Tectal glioma represents a distinct subgroup of brainstem tumors associated with a good (or favorable) prognosis. Effective treatment for hydrocephalus is essential; the tumor should be monitored by regular clinical examination and magnetic resonance imaging. Biopsy is warranted in cases with tumor progression.